OBJECTIVES: To describe employment status and work/activity impairment in individuals with HD by chorea severity and HD stage.

METHODS: Adults aged ≥18 years from Enroll-HD, a large, global, observational registry of individuals with/at risk of HD (2013–31 Oct 2020), were classified as non-HD, pre-manifest, or manifest HD. The manifest subpopulation was grouped by chorea severity using Total Maximal Chorea (TMC) scores and by HD stage using Total Functional Capacity (TFC) scores at baseline. Employment status and work productivity were assessed via the Work Productivity and Activity Impairment Questionnaire.

RESULTS: There were 4996 participants in the non-HD group, 5173 in the pre-manifest group, and 10,917 in the manifest group. The latter were classified by HD stage: TFC scores of 7–13 (early; n=7441), 3–6 (middle; n=2330), and 0–2 (late; n=1120). The proportion of unemployed participants increased from pre-manifest (24.3%), to early- (68.1%), middle- (98.4%), and late-stage (99.6%) HD. Among employed participants, overall activity impairment was similar in the pre-manifest and non-HD subpopulations (~5% in both groups), and increased as HD progressed (early, 31.5%; middle, 59.4%; late, 82.4%). Patients in the manifest subpopulation were also classified by chorea severity: TMC score 0–7, n=5055; 8–14, n=4374; 15–21, n=1310; and 22–28, n=164. As chorea severity increased, unemployment increased (71.4%, 80.2%, 92.3%, 96.3%, respectively) as well as overall HD-related work impairment (20.2%, 26.1%, 31.1%, 85.5%) and activity impairment (34.5%, 41.4%, 55.4%, 66.1%).

CONCLUSIONS: These results indicate increased work/activity impairment and unemployment when moving from pre-manifest to early-stage manifest HD, demonstrating substantial disease burden even during the earliest stages of HD, with increased burden as HD stage increased. These results support previous findings of the large societal impact of HD and suggest that chorea severity is a factor that correlates with work/activity impairment.